The studies proposed for the coming year will be divided into two primary areas of research. The first is the study of various glycolytic intermediates, especially glycerol, on the metabolism in preparations of brain tissue. These are designed to study the effects of various metabolites that have been shown previously to have special effects either in this tissue or others (e.g. glycerol effect in brain). The second area of effort is to elucidate the response of cultured fibroblast cell lines from normal and a CoA transferase-deficient patient to various energy-yielding substrates. These studies will involve the measurement of growth, oxidation rates, and intermediate accumulations in cells that are exposed to alternate (other than glucose) sources for oxidative energy production. BIBLIOGRAPHIC REFERENCES: Ozand, P.T., Tildon, J.T., Stevenson, J.H., Reed, W.D. and Cornblath, M.: The ability of exogenous alanine to lower blood beta hydroxybutyrate during development in rats. Am. J. Physiol. 230:1385 (1976). Tildon, J.T., Stevenson, J.H., Jr., and Ozand, P.T.: Mitochondrial glycerol kinase activity in rat brain. Biochem. J. 157:513 (1976).